Abstract
Data suggest that despite improved surgical outcomes for infants with hypoplastic left heart syndrome (HLHS), the past two decades have seen little change in parents’ decisions whether to choose surgery or palliative treatment without life-prolonging intervention. Data also suggest that doctors’ predictions of the choices they would make if their own infant were diagnosed with HLHS do not correlate with their predictions of surgical outcomes. Although previous studies have compared rates of surgery and palliative treatment without life-prolonging intervention over time, no studies have assessed changes in doctors’ attitudes. The current study used descriptive and quantitative statistics to compare responses from American pediatric cardiologists and congenital cardiac surgeons from studies conducted in 1999 and 2007. These doctors were asked what choice they believe they would make for their own affected infant. Comparison of responses from 1999 and 2007 showed no difference in the responses of cardiologists: 1999 (44 % surgery, 17 % palliative treatment, 40 % uncertain) versus 2007 (45 % surgery, 20 % palliative treatment, 35 % uncertain). Among surgeons, there was a non-statistically significant trend away from choosing surgery: 1999 (77 % surgery, 5 % palliative treatment, 18 % uncertain) versus 2007 (56 % surgery, 8 % palliative treatment, 36 % uncertain). In conclusion, these analyses suggest that despite improving surgical outcomes, doctors are no more likely to predict that they would choose surgery for their own hypothetical infant with HLHS. Further research is needed to determine what factors influence choice making in the care of infants with HLHS.
Similar content being viewed by others
References
Aeba R, Katogi T, Takeuchi S, Kawada S (1997) Long-term follow-up of surgical patients with single-ventricle physiology: prognostic anatomical determinants. Cardiovasc Surg 5:526–532
Allan LD, Apfel HD, Printz BF (1998) Outcome after prenatal diagnosis of the hypoplastic left heart syndrome. Heart 79:371–373
Ballweg JA, Dominguez TE, Ravishankar C, Kreutzer J, Marino BS, Bird GL, Gruber PJ, Wernovsky G, Gaynor JW, Nicolson SC, Spray TL, Tabbutt S (2007) A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction. J Thorac Cardiovasc Surg 134:297–303
Bove EL (1998) Current status of staged reconstruction for hypoplastic left heart syndrome. Pediatr Cardiol 19:308–315
Bove EL (1999) Surgical treatment for hypoplastic left heart syndrome. Jpn J Thorac Cardiovasc Surg 47:47–56
Bove EL, Lloyd TR (1996) Staged reconstruction for hypoplastic left heart syndrome: contemporary results. Ann Surg 224:387–394
Breymann T, Kirchner G, Blanz U, Cherlet E, Knobl H, Meyer H, Korfer R, Thies WR (1999) Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations. Eur J Cardiothorac Surg 16:117–124
Chang RK, Chen AY, Klitzner TS (2002) Clinical management of infants with hypoplastic left heart syndrome in the United States, 1988–1997. Pediatrics 110(2 Pt 1):292–298
Checchia PA, McGuire JK, Morrow S, Daher N, Huddleston C, Levy F (2006) A risk assessment scoring system predicts survival following the Norwood procedure. Pediatr Cardiol 27:62–66
Chrisant MR, Naftel DC, Drummond-Webb J, Chinnock R, Canter CE, Boucek MM, Boucek RJ, Hallowell SC, Kirklin JK, Morrow WR (2005) Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Heart Lung Transplant 24:576–582
Cohen DM, Allen HD (1997) New developments in the treatment of hypoplastic left heart syndrome. Curr Opin Cardiol 12:44–50
Cua CL, Thiagarajan RR, Gauvreau K, Lai L, Costello JM, Wessel DL, Del Nido PJ, Mayer JE Jr, Newburger JW, Laussen PC (2006) Early postoperative outcomes in a series of infants with hypoplastic left heart syndrome undergoing stage I palliation operation with either modified Blalock-Taussig shunt or right ventricle-to-pulmonary artery conduit. Pediatr Crit Care Med 7:238–244
Gutgesell HP, Gibson J (2002) Management of hypoplastic left heart syndrome in the 1990s. Am J Cardiol 89:842–846
Gutgesell HP, Massaro TA (1995) Management of hypoplastic left heart syndrome in a consortium of university hospitals. Am J Cardiol 76:809–811
Hehrlein FW, Yamamoto T, Orime Y, Bauer J (1998) Hypoplastic left heart syndrome: “Which is the best operative strategy?”. Ann Thorac Cardiovasc Surg 4:125–132
Ishino K, Stumper O, De Giovanni JJ, Silove ED, Wright JG, Sethia B, Brawn WJ, de Leval M (1999) The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg 117:920–930
Jaquiss RD, Siehr SL, Ghanayem NS, Hoffman GM, Fedderly RT, Cava JR, Mussatto KA, Tweddell JS (2006) Early cavopulmonary anastomosis after Norwood procedure results in excellent Fontan outcome. Ann Thorac Surg 82:1260–1265
Karamlou T, Diggs BS, Ungerleider RM, Welke KF (2010) Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. J Thorac Cardiovasc Surg 139:119–126 discussion 126–127
Kern JH, Hayes CJ, Michler RE, Gersony WM, Quaegebeur JM (1997) Survival and risk factor analysis for the Norwood procedure for hypoplastic left heart syndrome. Am J Cardiol 80:170–174
Kern JH, Hinton VJ, Nereo NE, Hayes CJ, Gersony WM (1998) Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome. Pediatrics 102:1148–1152
Kon AA (2005) Current outcomes and considerations in hypoplastic left heart syndrome. Curr Pediatr Rev 1:243–248
Kon AA (2008) Healthcare providers must offer palliative treatment to parents of neonates with hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 162:844–848
Kon AA, Ackerson L, Lo B (2003) Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome. Am J Cardiol 91:1506–1509
Kon AA, Ackerson L, Lo B (2004) How pediatricians counsel parents when no “best-choice” treatment exists: lessons to be learned from the hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 158:436–441
Kuebelbeck A (2003) Waiting with Gabriel: a story of cherishing a baby’s brief life. Loyola Press, Chicago
Lai L, Laussen PC, Cua CL, Wessel DL, Costello JM, del Nido PJ, Mayer JE, Thiagarajan RR (2007) Outcomes after bidirectional Glenn operation: Blalock–Taussig shunt versus right ventricle-to-pulmonary artery conduit. Ann Thorac Surg 83:1768–1773
Mahle WT, Visconti KJ, Freier MC, Kanne SM, Hamilton WG, Sharkey AM, Chinnock RE, Jenkins KJ, Isquith PK, Burns TG, Jenkins PC (2006) Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome. Pediatrics 117:e90–e397 Epub 15 December 2005
Prsa M, Holly CD, Carnevale FA, Justino H, Rohlicek CV (2010) Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics 125:e625–e630
Razzouk AJ, Chinnock RE, Gundry SR, Johnston JK, Larsen RL, Baum MF, Mulla NF, Bailey LL (1996) Transplantation as a primary treatment for hypoplastic left heart syndrome: intermediate-term results. Ann Thorac Surg 62:1–7
Sarajuuri A, Jokinen E, Puosi R, Eronen M, Mildh L, Mattila I, Valanne L, Lonnqvist T (2007) Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: related risk factor analysis. J Thorac Cardiovasc Surg 133:1524–1532
Stasik CN, Gelehrter S, Goldberg CS, Bove EL, Devaney EJ, Ohye RG (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 131:412–417
Tweddell JS, Ghanayem NS, Mussatto KA, Mitchell ME, Lamers LJ, Musa NL, Berger S, Litwin SB, Hoffman GM (2007) Mixed venous oxygen saturation monitoring after stage 1 palliation for hypoplastic left heart syndrome. Ann Thorac Surg 84:1301–1310
Vandvik IH, Forde R (2000) Ethical issues in parental decision making. An interview study of mothers of children with hypoplastic left heart syndrome. Acta Paediatr 89:1129–1133
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kon, A.A., Prsa, M. & Rohlicek, C.V. Choices Doctors Would Make If Their Infant Had Hypoplastic Left Heart Syndrome: Comparison of Survey Data From 1999 and 2007. Pediatr Cardiol 34, 348–353 (2013). https://doi.org/10.1007/s00246-012-0455-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-012-0455-9